MyEClinic
MyEClinic
Health Dictionary Find a Doctor

Temporal arteritis

For patient information, click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Synonyms and keywords: GCA; giant cell arteritis; cranial arteritis; Horton’s disease; Horton disease; Horton’s arteritis; Horton syndrome; Horton’s syndrome; Horton-Magath-Brown syndrome granulomatous arteritis; polymyalgia arteritica

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Overview

Temporal arteritis is an inflammatory disease of blood vessels (most commonly large and medium arteries of the head). It is therefore a form of vasculitis. The name comes from the most frequently involved vessel (temporal artery which branches from the external carotid artery of the neck). The alternative name (giant cell arteritis) reflects the type of inflammatory cell that is involved (as seen on biopsy).

Historical Perspective

The disease was first described in 1890 by Hutchinson. The histopathology of the disorder was first described in 1932 by Horton. Visual loss was first reported by Jennings in 1938. In 1957, Birkhead first described the use of steroids to prevent progression to blindness.

Classification

There is no established system for the classification of temporal arteritis.

Pathophysiology

Temporal arteritis is caused by transmural inflammation of elastic arteries. It is understood that temporal arteritis is the result of cell mediated immunity which arises as a response to endothelial injury and is antigen-driven disease with T-cell and macrophage activation in the elastic tissue in the arterial walls. The adventitia of the vessel is the initial site of immunologic injury. The activation of dendritic cells in the adventitia causes a production of chemokines that recruit CD4+ T helper cells. The CD4+ T helper cell convert in to Th17 cells which produce interleukin 17 and Th1 cells which produce interferon gamma. Giant cell are one of many inflammatory cells that are recruited and produce growth factor which narrows and obstructs the vessels. The concentric inflammation occurs in segments. Macrophages in the adventitia produce interleukin 6. While in the intima and media of the vessel, macrophages produce vascular endothelial growth factor (VEGF) and metalloproteinases which destroy the internal elastic lamina. An increased activated platelets express P-selectin which may cause vessel inflammation and thromboembolic events. Temporal arteritis arises from giant cells, which are fused monocytes cells that are normally involved in the body immune response. Because the disease involves only arteries with internal elastic lamina, the aortic arch and its branches are often involved. Intracranial arteries do not have internal elastic lamina and are not involved.

Causes

The cause of temporal arteritis has not been identified.

Differentiating Temporal Arteritis from Other Diseases

The differential of temporal arteritis include Takayasu arteritis, neurofibromatosis 1 and 2, fibromuscular dysplasia, Ehlers-Danlos syndrome, polymyalgia rheumatica (PMR), amyloidosis, and cerebral aneurysm.

Epidemiology and Demographics

Incidence of temporal arteritis ranges from approximately 0.5 to 27 cases per 100,000 people aged 50 years or older. The incidence of temporal arteritis increases with age; the median age at diagnosis is 76.7 years. Temporal arteritis commonly affects individuals older than 70 years of age. Temporal arteritis usually affects individuals of the white race. Black, Asians, and Hispanic individuals are less likely to develop temporal arteritis. Women are more commonly affected by temporal arteritis than men. The female to male ratio is approximately 3 to 1. The highest incidence of temporal arteritis is reported in Scandanavian countries at 32.7 per 100,000 people for people over 50 years of age.

Risk Factors

Common risk factors in the development of temporal arteritis may be environmental, genetic, and viral. Common risk factors in the development of temporal arteritis include age, hypertension, and thrombocytosis. Less common risk factors in the development of temporal arteritis include allele HLA-DRB1*04 genetic polymorphisms, varicella zoster virus infection, and smoking.

Natural History, Complications and Prognosis

The symptoms of temporal arteritis usually develop in the fifth decade of life, and start with symptoms such as headache, fatigue, jaw claudication, and vision problems. Common complications of temporal arteritis include blindness, aortic aneurysm, stroke, death, and side effects from steroid or immune-suppressing medications. Depending on the extent of the vessels involved in temporal arteritis at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor. If left untreated, 2% of patients with temporal arteritis may lead to death by MI or stroke.

Diagnosis

History and Symptoms

The hallmark of temporal arteritis is a headache. The most common symptoms of temporal arteritis include headache, jaw claudication, and fatigue. Common symptoms of temporal arteritis include visual symptoms, jaw claudication, headache, fatigue, and neck, shoulder, pelvic girdle pain. Less common symptoms of temporal arteritis include limb claudication, TIA, stroke, facial pain, bleeding gums, mouth sores, and joint pains.

Physical Examination

Patients with temporal arteritis usually appear in pain or normal. The presence of temporal tenderness on physical examination is highly suggestive of temporal arteritis. Ophthalmoscopic exam may be abnormal with findings of sludging of blood in retinal arterioles, optic disc may show chalky white pallor and edema, with or without splinter hemorrhages, posterior ischemic (retrobulbar) optic neuropathy, central retinal artery occlusion, branch of retinal artery occlusion, and choroidal ischemia. Tenderness upon palpation of the temporal region including erythema, nodularity, and thickening on affected side. Other findings include facial tenderness, diplopia, ptosis, nystagmus, internuclear ophthalmoplegia (INO), and pupillary abnormalities.

Laboratory Findings

The sedimentation rate is very high in most of the patients, but may be normal in approximately 20% of the cases. An elevated concentration of blood erythrocyte sedimentation rate (ESR) is suggestive of temporal arteritis. Laboratory findings consistent with the diagnosis of temporal arteritis include normochromic anemia, deceased albumin level, elevated alkaline phosphatase, and elevated C-reactive protein. Some patients with temporal arteritis may have elevated concentration of interleukin-6, which is usually suggestive of progression.

CT

Contrast enhanced brain CT is generally negative in this disorder.

MRI

A contrast enhanced brain MRI is generally negative in this disorder.

Ultrasound

A radiological examination of the temporal artery with ultrasound yields a halo sign. Ultrasound of the temporal artery may be helpful in the diagnosis of temporal arteritis. Findings on an ultrasound suggestive of temporal arteritis include hypoechoic halo around the lumen of the temporal artery. The halo is suggestive of edema in the temporal artery wall.

Other Diagnostic Studies

The gold standard for diagnosing temporal arteritis is biopsy, which involves removing of a small part of the vessel and examining it microscopically for giant cells infiltrating the tissue. Since the blood vessels are involved in a patchy pattern, there may be unaffected areas on the vessel and the biopsy might have been taken from these parts. So, a negative result does not definitely rule out the diagnosis. Findings diagnostic of temporal arteritis include skip lesions and normal intervening segments, intimal thickening, with prominent cellular infiltration, lymphocytes in the internal or external elastic lamina or adventitia, areas of necrosis may be present in the arterial wall, granulomas containing multinucleated histiocytic and foreign body giant cells, helper T-cell lymphocytes, plasma cells, and fibroblasts. Risks of temporal artery biopsy are temporary or permanent damage to the temporal branch of the facial nerve, infection, bleeding, hematoma, and dehiscence.

Treatment

Medical Therapy

The mainstay of treatment for temporal arteritis is corticosteroids. Damage may be irreversible if treatment is delayed beyond 48 hours. Oral prednisone 40-60 mg/day should be started, with a temporal artery biopsy performed within 1 week. Prednisone doses of 80-100 mg/day have been suggested for patients with visual or neurologic symptoms of temporal arteritis and follow-up care within 72 hours after starting therapy should be arranged. Patients with acute visual changes from temporal arteritis can be started on intravenous (IV) methylprednisolone at a dose of 1,000 mg daily for 3 days. Initial high-dose IV corticosteroid, methylprednisolone, treatment 15 mg/kg of ideal body weight/day may reduce remission rates. Use of low-dose aspirin (81 mg) for prevention of visual loss and stroke is recommended in temporal arteritis. Improvement of systemic symptoms occurs within 72 hours after initiation of therapy. The elevation in erythrocyte sedimentation rate and ischemic symptoms such as headache and jaw claudication improve over several days. High-dose steroid therapy should be maintained long enough for symptoms to resolve and then be tapered slowly to the lowest dose required to suppress symptoms. Long-term corticosteroid therapy complications include diabetes mellitus, vertebral compression fractures, steroid myopathy, steroid psychosis, and immunosuppression related infections. Tocilizumab has been approved for use in rheumatoid arthritis and is now approved for use in temporal arteritis.

Surgery

Surgical intervention is not recommended for the management of temporal arteritis.

Primary Prevention

There is no known prevention for temporal arteritis.

References

Template:WH Template:WS

Historical Perspective

Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Overview

The disease was first described in 1890 by Hutchinson. The histopathology of the disorder was first described in 1932 by Horton. Visual loss was first reported by Jennings in 1938. In 1957, Birkhead first described the use of steroids to prevent progression to blindness.

Historical Perspective

Discovery

  • Temporal arteritis was first discovered by Hutchison, in 1890.
  • Temporal arteritis was described by Horton et al, in 1932.

Landmark Events in the Development of Treatment Strategies

  • In 1957, steroid therapy to prevent progression to vision loss was described by Birkhead et al to treat temporal arteritis.[1].

References

  1. ↑ BIRKHEAD NC, WAGENER HP, SHICK RM (1957). “Treatment of temporal arteritis with adrenal corticosteroids; results in fifty-five cases in which lesion was proved at biopsy”. J Am Med Assoc. 163 (10): 821–7. PMID 13405740.

Template:WikiDoc Sources

Classification

Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Overview

There is no established system for the classification of temporal arteritis.

Classification

There is no established system for the classification of temporal arteritis.

References

Template:WikiDoc Sources

Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Overview

Temporal arteritis is caused by transmural inflammation of elastic arteries. It is understood that temporal arteritis is the result of cell mediated immunity which arises as a response to endothelial injury and is antigen-driven disease with T-cell and macrophage activation in the elastic tissue in the arterial walls. The adventitia of the vessel is the initial site of immunologic injury. The activation of dendritic cells in the adventitia causes a production of chemokines that recruit CD4+ T helper cells. The CD4+ T helper cell convert in to Th17 cells which produce interleukin 17 and Th1 cells which produce interferon gamma. Giant cell are one of many inflammatory cells that are recruited and produce growth factor which narrows and obstructs the vessels. The concentric inflammation occurs in segments. Macrophages in the adventitia produce interleukin 6. While in the intima and media of the vessel, macrophages produce vascular endothelial growth factor (VEGF) and metalloproteinases which destroy the internal elastic lamina. An increased activated platelets express P-selectin which may cause vessel inflammation and thromboembolic events. Temporal arteritis arises from giant cells, which are fused monocytes cells that are normally involved in the body immune response. Because the disease involves only arteries with internal elastic lamina, the aortic arch and its branches are often involved. Intracranial arteries do not have internal elastic lamina and are not involved.

Pathophysiology

Pathogenesis

Commonly involved sites:[9][10][11][12]

  • External vertebral arteries: It is less common though for the disease to extend more than 5 mm beyond the dural penetration.

Less commonly involved sites:[11]

Genetics

Associated Conditions

Microscopic Pathology

Giant cell arteritis-low mag Source: Case courtesy of By Nephron [CC BY-SA 3.0, from Wikimedia Commons


Video

{{#ev:youtube|UrGsVfLbnA0}}

{{#ev:youtube|wBh4bI7Do3s}}

References

  1. ↑ Salvarani, C.; Pipitone, N.; Boiardi, L.; Hunder, G. G (2007). “Do we need treatment with tumour necrosis factor blockers for giant cell arteritis?”. Annals of the Rheumatic Diseases. 67 (5): 577–579. doi:10.1136/ard.2007.086330. ISSN 0003-4967.
  2. ↑ Pache M, Kaiser HJ, Haufschild T, Lübeck P, Flammer J (2002). “Increased endothelin-1 plasma levels in giant cell arteritis: a report on four patients”. Am J Ophthalmol. 133 (1): 160–2. PMID 11755863.
  3. ↑ Patel SJ, Lundy DC (2002). “Ocular manifestations of autoimmune disease”. Am Fam Physician. 66 (6): 991–8. PMID 12358224.
  4. ↑ 4.0 4.1 Wang AL, Raven ML, Surapaneni K, Albert DM (2017). “Studies on the Histopathology of Temporal Arteritis”. Ocul Oncol Pathol. 3 (1): 60–65. doi:10.1159/000449466. PMC 5318845. PMID 28275606.
  5. ↑ Samson, Maxime; Corbera-Bellalta, Marc; Audia, Sylvain; Planas-Rigol, Ester; Martin, Laurent; Cid, Maria Cinta; Bonnotte, Bernard (2017). “Recent advances in our understanding of giant cell arteritis pathogenesis”. Autoimmunity Reviews. 16 (8): 833–844. doi:10.1016/j.autrev.2017.05.014. ISSN 1568-9972.
  6. ↑ 6.0 6.1 Eberhardt RT, Dhadly M (2007). “Giant cell arteritis: diagnosis, management, and cardiovascular implications”. Cardiol Rev. 15 (2): 55–61. doi:10.1097/01.crd.0000218853.05856.b6. PMID 17303991.
  7. ↑ Diaz, Vicente A.; DeBroff, Brian M.; Sinard, John (2005). “Comparison of Histopathologic Features, Clinical Symptoms, and Erythrocyte Sedimentation Rates in Biopsy-Positive Temporal Arteritis”. Ophthalmology. 112 (7): 1293–1298. doi:10.1016/j.ophtha.2005.02.016. ISSN 0161-6420.
  8. ↑ Goodwin JS (1992). “Progress in gerontology: polymyalgia rheumatica and temporal arteritis”. J Am Geriatr Soc. 40 (5): 515–25. PMID 1634709.
  9. ↑ Waldman, Corey W.; Waldman, Steven D.; Waldman, Reid A. (2013). “Giant Cell Arteritis”. Medical Clinics of North America. 97 (2): 329–335. doi:10.1016/j.mcna.2012.12.006. ISSN 0025-7125.
  10. ↑ Pineles, Stacy L.; Arnold, Anthony C. (2007). “Giant Cell Arteritis”. International Ophthalmology Clinics. 47 (4): 105–119. doi:10.1097/IIO.0b013e318157fb08. ISSN 0020-8167.
  11. ↑ 11.0 11.1 Liozon, E.; Ly, K.-H.; Robert, P.-Y. (2013). “Manifestations ophtalmologiques de la maladie de Horton”. La Revue de Médecine Interne. 34 (7): 421–430. doi:10.1016/j.revmed.2013.02.030. ISSN 0248-8663.
  12. ↑ Wilkinson IM, Russell RW (1972). “Arteries of the head and neck in giant cell arteritis. A pathological study to show the pattern of arterial involvement”. Arch Neurol. 27 (5): 378–91. PMID 5078894.
  13. ↑ Liozon E, Ouattara B, Rhaiem K, Ly K, Bezanahary H, Loustaud V; et al. (2009). “Familial aggregation in giant cell arteritis and polymyalgia rheumatica: a comprehensive literature review including 4 new families”. Clin Exp Rheumatol. 27 (1 Suppl 52): S89–94. PMID 19646354.
  14. ↑ Palomino-Morales R, Torres O, Vazquez-Rodriguez TR, Morado IC, Castañeda S, Callejas-Rubio JL; et al. (2009). “Association between toll-like receptor 4 gene polymorphism and biopsy-proven giant cell arteritis”. J Rheumatol. 36 (7): 1501–6. doi:10.3899/jrheum.081286. PMID 19531762.
  15. ↑ Caylor TL, Perkins A (2013). “Recognition and management of polymyalgia rheumatica and giant cell arteritis”. Am Fam Physician. 88 (10): 676–84. PMID 24364483.
  16. ↑ Salvarani, Carlo; Cantini, Fabrizio; Hunder, Gene G (2008). “Polymyalgia rheumatica and giant-cell arteritis”. The Lancet. 372 (9634): 234–245. doi:10.1016/S0140-6736(08)61077-6. ISSN 0140-6736.
  17. ↑ Alvarez-Lafuente, R (2005). “Human parvovirus B19, varicella zoster virus, and human herpes virus 6 in temporal artery biopsy specimens of patients with giant cell arteritis: analysis with quantitative real time polymerase chain reaction”. Annals of the Rheumatic Diseases. 64 (5): 780–782. doi:10.1136/ard.2004.025320. ISSN 0003-4967.
  18. ↑ Russo MG, Waxman J, Abdoh AA, Serebro LH (1995). “Correlation between infection and the onset of the giant cell (temporal) arteritis syndrome. A trigger mechanism?”. Arthritis Rheum. 38 (3): 374–80. PMID 7880192.
  19. ↑ Gilden, Don; Nagel, Maria (2015). “Varicella Zoster Virus in Temporal Arteries of Patients With Giant Cell Arteritis”. Journal of Infectious Diseases. 212 (suppl 1): S37–S39. doi:10.1093/infdis/jiu542. ISSN 0022-1899.
  20. ↑ Pountain G, Hazleman B (1995). “ABC of rheumatology. Polymyalgia rheumatica and giant cell arteritis”. BMJ. 310 (6986): 1057–9. PMC 2549437. PMID 7728064.
  21. ↑ Weyand CM, Fulbright JW, Hunder GG, Evans JM, Goronzy JJ (2000). “Treatment of giant cell arteritis: interleukin-6 as a biologic marker of disease activity”. Arthritis Rheum. 43 (5): 1041–8. doi:10.1002/1529-0131(200005)43:5<1041::AID-ANR12>3.0.CO;2-7. PMID 10817557.
  22. ↑ Liozon E, Ly KH, Robert PY (2013). “[Ocular complications of giant cell arteritis]”. Rev Med Interne. 34 (7): 421–30. doi:10.1016/j.revmed.2013.02.030. PMID 23523078.


Template:WikiDoc Sources

Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Overview

The cause of temporal arteritis has not been identified. To review risk factors for the development of temporal arteritis, click here.

Causes

The cause of temporal arteritis has not been identified. To review risk factors for the development of temporal arteritis, click here.

References

Template:WikiDoc Sources

Differentiating Temporal Arteritis from other Diseases

Differentiating Temporal Arteritis from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Differentiating Temporal Arteritis from other Diseases

The following list is a differential diagnosis of disorders that temporal arteritis should be distinguished from.

Differentiating Temporal arteritis from other diseases that may cause arthritis and rash

Abbreviations: ANA: Antinuclear antibody, RF: Rheumatoid factor, Anti-CCp: Anti-cyclic citrullinated protein antibody, Anti U1RNP: Anti-U1 ribonucleoprotein antibodies , Anti Sm : Anti-Sm antibodies, Anti Ro: Anti Ro antibody also called anti-Sjögren’s-syndrome-related antigen A antibody, Anti-dsDNA: Anti-double stranded DNA.

Disease Arthritis Auto-antibodies Raynaud phenomenon Rash pattern Distinguishing/specific features
Polyarthritis Tenderness Edema Deformity /Erosion Pattern ANA RF Anti-CCp Anti U1RNP Anti Sm Anti Ro Anti-dsDNA
Vasculitis Temporal arteritis[1] – + + – Distal extremity – – – – – – – – Rare Involvement of cranial branches of arteries, visual loss
Takayasu[2] – +/- +/- – Transient extremity – – – – – – – Erythema nodosum, pyoderma gangrenosum Absent or weak peripheral pulse
Poly-arteritis nodosa[3] – +/- – – General and mild – – – – – – – Tender erythematous nodules, purpura, livedo reticularis, bullous or vesicular eruption Testicular pain or tenderness and neuropathies
Systemic lupus erythematosus[4] + + + – Small joints ↑ – – – ↑ ↑ – + Malar rash and photosensitivity
Rheumatoid arthritis (RA)[5] + + + + Small and large joints – ↑↑ ↑↑ – – – – + Subcutaneous nodules Erosive arthropathy
Rhupus[6] + + + + Small and large joints ↑ ↑ ↑ ↑ ↑ – ↑ + Malar rash and photosensitivity Erosive arthropathy
Mixed connective tissue disease (MCTD)[7] – – – + Small and large joints – ↑↑ ↑ – – – – + Cutaneous eruptions, gottron’s papules, photodistributed erythema, poikiloderma, and calcinosis cutis Overlapping features of SLE, systemic sclerosis (SSc), and polymyositis (PM) that lead to more than one diagnosis
Undifferentiated connective tissue disease (UCTD)[8] + – – – Lower extremity ↑ ↑ – – ↑ – – + Erythematous macules, patches, or papules with delicate scale Multiple connective tissue diseases with no enough criteria for a single diagnosis
Systemic sclerosis (SSc)[9] +/- + + +/- Lower extremity ↑↑ – – – ↑ – ↑ + Hyperkeratosis, edema, and erythema Sclerodactyly, Telangiectasias, Calcinosis, Malignant hypertension, acute renal failure
Sjögren’s syndrome[10] +/- +/- – – Lower extremity, axiallary creases ↑ – – – ↑ ↑ – – Xerosis, scaly skin, annular erythema Keratoconjunctivitis sicca
Behçet’s syndrome[11] +/- +/- +/- – medium and large joints – – – – – – – – Recurrent and usually painful mucocutaneous ulcers, acneiform lesions, papulo-vesiculo-pustular eruptions, superficial thrombophlebitis Male dominancy
Kikuchi’s disease[12] – +/- – – medium and large joints ↑/↓ – – – – – – – Transient skin rashes, malar rash, erythematous macules, patches, papules, or plaques May be associated with SLE
Serum sickness[13] + + +/- – General – – – – – – – – Pruritic rash, urticaria and/or serpiginous macular rash Self-limited
Psoriatic arthritis[14] – – – – Small and large joints – – – – – – – – Psoriasis and onychodystrophy Dactylitis (sausage digits)
Human parvovirus B19 infection[15] + + – – Small joints – – – – – – – – Erythematous rashes Rare in adults, fifth’s disease in children

References

  1. ↑ Bablekos GD, Michaelides SA, Karachalios GN, Nicolaou IN, Batistatou AK, Charalabopoulos KA (2006). “Pericardial involvement as an atypical manifestation of giant cell arteritis: report of a clinical case and literature review”. Am. J. Med. Sci. 332 (4): 198–204. PMID 17031245.
  2. ↑ Lupi-Herrera E, Sánchez-Torres G, Marcushamer J, Mispireta J, Horwitz S, Vela JE (1977). “Takayasu’s arteritis. Clinical study of 107 cases”. Am. Heart J. 93 (1): 94–103. PMID 12655.
  3. ↑ Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, Bienvenu B, Mouthon L, Guillevin L (2010). “Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database”. Arthritis Rheum. 62 (2): 616–26. doi:10.1002/art.27240. PMID 20112401.
  4. ↑ Ehmke TA, Cherian JJ, Wu ES, Jauregui JJ, Banerjee S, Mont MA (2014). “Treatment of osteonecrosis in systemic lupus erythematosus: a review”. Curr Rheumatol Rep. 16 (9): 441. doi:10.1007/s11926-014-0441-8. PMID 25074031.
  5. ↑ Lee DM, Weinblatt ME (2001). “Rheumatoid arthritis”. Lancet. 358 (9285): 903–11. doi:10.1016/S0140-6736(01)06075-5. PMID 11567728.
  6. ↑ Panush RS, Edwards NL, Longley S, Webster E (1988). “‘Rhupus’ syndrome”. Arch. Intern. Med. 148 (7): 1633–6. PMID 3382309.
  7. ↑ Cappelli S, Bellando Randone S, Martinović D, Tamas MM, Pasalić K, Allanore Y, Mosca M, Talarico R, Opris D, Kiss CG, Tausche AK, Cardarelli S, Riccieri V, Koneva O, Cuomo G, Becker MO, Sulli A, Guiducci S, Radić M, Bombardieri S, Aringer M, Cozzi F, Valesini G, Ananyeva L, Valentini G, Riemekasten G, Cutolo M, Ionescu R, Czirják L, Damjanov N, Rednic S, Matucci Cerinic M (2012). ““To be or not to be,” ten years after: evidence for mixed connective tissue disease as a distinct entity”. Semin. Arthritis Rheum. 41 (4): 589–98. doi:10.1016/j.semarthrit.2011.07.010. PMID 21959290.
  8. ↑ Alarcón GS, Williams GV, Singer JZ, Steen VD, Clegg DO, Paulus HE, Billingsley LM, Luggen ME, Polisson RP, Willkens RF (1991). “Early undifferentiated connective tissue disease. I. Early clinical manifestation in a large cohort of patients with undifferentiated connective tissue diseases compared with cohorts of well established connective tissue disease”. J. Rheumatol. 18 (9): 1332–9. PMID 1757934.
  9. ↑ LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Rowell N, Wollheim F (1988). “Scleroderma (systemic sclerosis): classification, subsets and pathogenesis”. J. Rheumatol. 15 (2): 202–5. PMID 3361530.
  10. ↑ Roguedas AM, Misery L, Sassolas B, Le Masson G, Pennec YL, Youinou P (2004). “Cutaneous manifestations of primary Sjögren’s syndrome are underestimated”. Clin. Exp. Rheumatol. 22 (5): 632–6. PMID 15485020.
  11. ↑ Tunç R, Uluhan A, Melikoğlu M, Ozyazgan Y, Ozdoğan H, Yazici H (2001). “A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet’s syndrome”. Clin. Exp. Rheumatol. 19 (5 Suppl 24): S45–7. PMID 11760398.
  12. ↑ Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M (2007). “Kikuchi-Fujimoto Disease: analysis of 244 cases”. Clin. Rheumatol. 26 (1): 50–4. doi:10.1007/s10067-006-0230-5. PMID 16538388.
  13. ↑ Kunnamo I, Kallio P, Pelkonen P, Viander M (1986). “Serum-sickness-like disease is a common cause of acute arthritis in children”. Acta Paediatr Scand. 75 (6): 964–9. PMID 3564980.
  14. ↑ Oriente P, Biondi-Oriente C, Scarpa R (1994). “Psoriatic arthritis. Clinical manifestations”. Baillieres Clin Rheumatol. 8 (2): 277–94. PMID 8076388.
  15. ↑ Kaufmann J, Buccola JM, Stead W, Rowley C, Wong M, Bates CK (2007). “Secondary symptomatic parvovirus B19 infection in a healthy adult”. J Gen Intern Med. 22 (6): 877–8. doi:10.1007/s11606-007-0173-9. PMC 2219874. PMID 17384979.


Template:WikiDoc Sources

Epidemiology and Demographics

Epidemiology and Demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Overview

Incidence of temporal arteritis ranges from approximately 0.5 to 27 cases per 100,000 people aged 50 years or older. The incidence of temporal arteritis increases with age; the median age at diagnosis is 76.7 years. Temporal arteritis commonly affects individuals older than 70 years of age. Temporal arteritis usually affects individuals of the white race. Black, Asians, and Hispanic individuals are less likely to develop temporal arteritis. Women are more commonly affected by temporal arteritis than men. The female to male ratio is approximately 3 to 1. The highest incidence of temporal arteritis is reported in Scandanavian countries at 32.7 per 100,000 people for people over 50 years of age.

Epidemiology and Demographics

Incidence

  • Incidence of temporal arteritis ranges from approximately 0.5 to 27 cases per 100,000 people aged 50 years or older.[1]

Age

  • The incidence of temporal arteritis increases with age; the median age at diagnosis is 76.7 years.[2]
  • Temporal arteritis commonly affects individuals older than 70 years of age.[3][4]

Race

  • Temporal arteritis usually affects individuals of the white race. Black, Asians, and Hispanic individuals are less likely to develop temporal arteritis.[5][6]

Gender

  • Women are more commonly affected by temporal arteritis than men. The female to male ratio is approximately 3 to 1.[5]

Region

Developed Countries

  • The highest incidence of temporal arteritis is reported in Scandanavian countries at 32.7 per 100,000 people for people over 50 years of age.[7]

References

  1. ↑ Goodwin JS (1992). “Progress in gerontology: polymyalgia rheumatica and temporal arteritis”. J Am Geriatr Soc. 40 (5): 515–25. PMID 1634709.
  2. ↑ Kermani TA, Schäfer VS, Crowson CS, Hunder GG, Gabriel SE, Matteson EL; et al. (2010). “Increase in age at onset of giant cell arteritis: a population-based study”. Ann Rheum Dis. 69 (4): 780–1. doi:10.1136/ard.2009.111005. PMID 19854712.
  3. ↑ Salvarani C, Crowson CS, O’Fallon WM, Hunder GG, Gabriel SE (2004). “Reappraisal of the epidemiology of giant cell arteritis in Olmsted County, Minnesota, over a fifty-year period”. Arthritis Rheum. 51 (2): 264–8. doi:10.1002/art.20227. PMID 15077270.
  4. ↑ Gonzalez-Gay MA, Miranda-Filloy JA, Lopez-Diaz MJ, Perez-Alvarez R, Gonzalez-Juanatey C, Sanchez-Andrade A; et al. (2007). “Giant cell arteritis in northwestern Spain: a 25-year epidemiologic study”. Medicine (Baltimore). 86 (2): 61–8. doi:10.1097/md.0b013e31803d1764. PMID 17435586.
  5. ↑ 5.0 5.1 Gonzalez-Gay MA, Vazquez-Rodriguez TR, Lopez-Diaz MJ, Miranda-Filloy JA, Gonzalez-Juanatey C, Martin J; et al. (2009). “Epidemiology of giant cell arteritis and polymyalgia rheumatica”. Arthritis Rheum. 61 (10): 1454–61. doi:10.1002/art.24459. PMID 19790127.
  6. ↑ Artal NM, Rodriguez M, Luna JD, Reviglio VE, Cuello O, Muiñ JC; et al. (2002). “Giant cell arteritis in a Hispanic population”. Ophthalmology. 109 (10): 1757, discussion 1757. PMID 12359586.
  7. ↑ Smeeth, L (2006). “Incidence of diagnosed polymyalgia rheumatica and temporal arteritis in the United Kingdom, 1990-2001”. Annals of the Rheumatic Diseases. 65 (8): 1093–1098. doi:10.1136/ard.2005.046912. ISSN 0003-4967.

Template:WikiDoc Sources

Risk Factors

Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Overview

Common risk factors in the development of temporal arteritis may be environmental, genetic, and viral. Common risk factors in the development of temporal arteritis include age, hypertension, and thrombocytosis. Less common risk factors in the development of temporal arteritis include allele HLA-DRB1*04 genetic polymorphisms, varicella zoster virus infection, and smoking.

Risk Factors

The risk factors of temporal arteritis are as follows:

Common Risk Factors

Less Common Risk Factors

References

  1. ↑ Nesher G, Berkun Y, Mates M, Baras M, Nesher R, Rubinow A; et al. (2004). “Risk factors for cranial ischemic complications in giant cell arteritis”. Medicine (Baltimore). 83 (2): 114–22. PMID 15028965.
  2. ↑ González-Gay MA, García-Porrúa C, Llorca J, Hajeer AH, Brañas F, Dababneh A; et al. (2000). “Visual manifestations of giant cell arteritis. Trends and clinical spectrum in 161 patients”. Medicine (Baltimore). 79 (5): 283–92. PMID 11039076.
  3. ↑ Salvarani C, Cimino L, Macchioni P, Consonni D, Cantini F, Bajocchi G; et al. (2005). “Risk factors for visual loss in an Italian population-based cohort of patients with giant cell arteritis”. Arthritis Rheum. 53 (2): 293–7. doi:10.1002/art.21075. PMID 15818722.
  4. ↑ Mahr, A.; Aouba, A.; Richebé, P.; Gonzalez-Chiappe, S. (2017). “Épidémiologie et histoire naturelle de l’artérite à cellules géantes (Horton)”. La Revue de Médecine Interne. 38 (10): 663–669. doi:10.1016/j.revmed.2017.03.007. ISSN 0248-8663.

Template:WikiDoc Sources

Natural History, Complications and Prognosis

Natural History, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Overview

The symptoms of temporal arteritis usually develop in the fifth decade of life, and start with symptoms such as headache, fatigue, jaw claudication, and vision problems. Common complications of temporal arteritis include blindness, aortic aneurysm, stroke, death, and side effects from steroid or immune-suppressing medications. Depending on the extent of the vessels involved in temporal arteritis at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor. If left untreated, 2% of patients with temporal arteritis may develop MI or stroke leading to death.

Natural History, Complications, and Prognosis

Natural History

  • If left untreated, 2% of patients with temporal arteritis may lead to death by MI or stroke.[2]

Complications

Prognosis

  • Depending on the extent of the vessels involved in temporal arteritis at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor.

References

  1. ↑ Wiszniewska, Malgorzata; Devuyst, Gerald; Bogousslavsky, Julien (2007). “Giant Cell Arteritis as a Cause of First-Ever Stroke”. Cerebrovascular Diseases. 24 (2–3): 226–230. doi:10.1159/000104482. ISSN 1015-9770.
  2. ↑ Tomasson, Gunnar; Peloquin, Christine; Mohammad, Aladdin; Love, Thorvardur J.; Zhang, Yuqing; Choi, Hyon K.; Merkel, Peter A. (2014). “Risk for Cardiovascular Disease Early and Late After a Diagnosis of Giant-Cell Arteritis”. Annals of Internal Medicine. 160 (2): 73–80. doi:10.7326/M12-3046. ISSN 0003-4819.
  3. ↑ Solans-Laqué, Roser; Bosch-Gil, Josep Angel; Molina-Catenario, Carlos A.; Ortega-Aznar, Arantxa; Alvarez-Sabin, José; Vilardell-Tarres, Miguel (2008). “Stroke and Multi-Infarct Dementia as Presenting Symptoms of Giant Cell Arteritis”. Medicine. 87 (6): 335–344. doi:10.1097/MD.0b013e3181908e96. ISSN 0025-7974.
  4. ↑ Taylor S, Lightman S (2003). “The eye in cardiac and cardiovascular disease”. Hosp Med. 64 (5): 299–301. PMID 12789741.
  5. ↑ Smulders YM, Verhagen DW (2008). “Giant cell arteritis causing aortic dissection and acute hypertension”. BMJ. 337: a426. doi:10.1136/bmj.39503.769225.BE. PMID 18614463.
  6. ↑ Scola, Christopher J.; Li, Cuizhen; Upchurch, Katherine S. (2008). “Mesenteric Involvement in Giant Cell Arteritis. An Underrecognized Complication?”. Medicine. 87 (1): 45–51. doi:10.1097/MD.0b013e3181646118. ISSN 0025-7974.

Template:WikiDoc Sources

Diagnosis

Diagnosis

Diagnostic Study of Choice | History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case #1

Template:Diseases of the musculoskeletal system and connective tissue


de:Arteriitis cranialis


Template:WikiDoc Sources

Looking for the patient version?

Back to the patient-friendly article

Imprint

Privacy Policy

Terms and Conditions

© 2026 MyEClinic – IFTM Institut für Telematik in der Medizin GmbH